Paediatric phaeochromocytoma and paraganglioma: A clinical update

Author:

Nazari Matthew A.1ORCID,Jha Abhishek1,Kuo Mickey J. M.12,Patel Mayank1,Prodanov Tamara1,Rosenblum Jared S.1,Talvacchio Sara1,Derkyi Alberta1,Charles Kailah1,Pacak Karel1

Affiliation:

1. Developmental Endocrinology, Metabolism, Genetics and Endocrine Oncology Affinity Group, Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA

2. Medical Genetics Branch, National Human Genome Research Institute National Institutes of Health Bethesda Maryland USA

Abstract

AbstractPaediatric phaeochromocytomas and paragangliomas (PPGLs), though rare tumours, are associated with significant disability and death in the most vulnerable of patients early in their lives. However, unlike cryptogenic and insidious disease states, the clinical presentation of paediatric patients with PPGLs can be rather overt, allowing early diagnosis, granted that salient findings are recognized. Additionally, with prompt and effective intervention, prognosis is favourable if timely intervention is implemented. For this reason, this review focuses on four exemplary paediatric cases, succinctly emphasizing the now state‐of‐the‐art concepts in paediatric PPGL management.

Publisher

Wiley

Subject

Endocrinology, Diabetes and Metabolism,Endocrinology

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